page 1 First Aid Express 2016 workbook: NEUROLOGY Neurology How to Use the Workbook with the Videos Using this table as a guide, read the Facts in First Aid for the USMLE Step 1 2016, watch the corresponding First Aid Express 2016 videos, and then answer the workbook questions. Facts in First Aid for the USMLE Step 1 2016 Corresponding First Aid Express 2015 videos Workbook questions 450.1–452.2 Embryology (2 videos) 1–3 453.1–482.1 Anatomy & Physiology (12 videos) 4–38 482.2–490.1 Ophthalmology (3 videos) 39–43 491.1–498.1 Pathology (6 videos) 44–48 498.2–505.6 Pharmacology (4 videos) 49–52 Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 2 First Aid Express 2016 workbook: NEUROLOGY Questions EMBRYOLOGY 1. What maternal disease is associated with anencephaly? (p 451) ___________________________ 2. What are the sensory abnormalities in syringomyelia? (p 452) ____________________________ ______________________________________________________________________________ 3. Which branchial arches form the posterior 1/3 of the tongue? (p 452) ______________________ ANATOMY AND PHYSIOLOGY 4. Match the cell type with its characteristic. (p 453) _____ A. Astrocytes _____ B. Microglia _____ C. Neurons _____ D. Schwann cells 5. 1. 2. 3. 4. Undergo Wallerian degeneration Affected in an acoustic neuroma Form multinucleated giant cells Maintain blood-brain barrier _____________(Oligodendroglia/Schwann cells) are destroyed in Gullain-Barré syndrome; _____________(oligodendroglia/Schwann cells) are destroyed in multiple sclerosis. (pp 453-454) 6. Match the disease with its alteration in the neurotransmitter. (p 455) _____ A. Alzheimer disease _____ B. Anxiety _____ C. Depression _____ D. Huntington disease 7. 1. 2. 3. 4. Decrease in acetylcholine Decrease in dopamine Increase in dopamine Increase in norepinephrine Which substances cross the blood-brain barrier quickly? Which substances cross it slowly? (p 455) ______________________________________________________________________________ ______________________________________________________________________________ 8. Match the area of the hypothalamus with its function. (p 456) _____ A. Anterior hypothalamus _____ B. Lateral area _____ C. Paraventricular nucleus _____ D. Posterior hypothalamus _____ E. Suprachiasmatic nucleus _____ F. Supraoptic nucleus _____ G. Ventromedial area 1. 2. 3. 4. 5. 6. 7. Circadian rhythm Cooling Heating Hunger Makes ADH Makes oxytocin Satiety 9. Ascending sensory information from the body reaches the __________ (VPL/VPM) of the thalamus, and sensory information from the face reaches the ____________ (VPL/VPM). (p 458) 10. Decreases in the activity of tuberoinfundibular pathway lead to increases in what secretory protein? (p 458) ________________________________________________________________________ Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 3 First Aid Express 2016 workbook: NEUROLOGY 11. In the basal ganglia, the direct pathway utilizes the ____ (D1/D2) receptor and __________ (facilitates/inhibits) movement. The indirect pathway utilizes the ___ (D1/D2) receptor and ____________ (facilitates/inhibits) movement. (p 460) 12. How does loss of dopamine in Parkinson disease affect the excitatory pathway? How does it affect the inhibitory pathway? (p 460) ____________________________________________________ ______________________________________________________________________________ 13. What are the cardinal features of Parkinson disease? (p 461) _____________________________ ______________________________________________________________________________ 14. Which nucleus is affected in Parkinson disease? In hemiballismus? In Huntington disease? (p 461) ________________________________________________________________________ 15. Define the following terms. (p 461) A. Athetosis __________________________________________________________________ B. Chorea ___________________________________________________________________ C. Dystonia __________________________________________________________________ D. Myoclonus ________________________________________________________________ 16. Fluent aphasia with impaired comprehension describes __________ (Broca/Wernicke) aphasia, whereas nonfluent aphasia with intact comprehension describes __________ (Broca/Wernicke) aphasia. (p 463) 17. Match the area of a brain lesion with its clinical effect(s). (p 464) _____ A. Anterograde amnesia _____ B. Contralateral hemiballismus _____ C. Deficits in concentration, orientation, and judgment _____ D. Eyes look away from side of lesion _____ E. Eyes look toward side of lesion _____ F. Intention tremor and limb ataxia _____ G. Klüver-Bucy syndrome _____ H. Reduced levels of arousal and wakefulness _____ I. Hemispatial neglect syndrome _____ J. Tremor at rest, chorea, or athetosis _____ K. Truncal ataxia and dysarthria _____ L. Wernicke-Korsakoff syndrome _____M. Agraphia, acalculia, finger agnosia, left-right disorientation 18. 1.Amygdala 2.Basal ganglia 3.Cerebellar hemisphere 4.Cerebellar vermis 5.Frontal eye fields 6.Frontal lobe 7.Hippocampus 8.Mamillary bodies 9.PPRF 10.Dominant parietal cortex 11.Nondominant parietal cortex 12.Subthalamic nucleus 13. Midbrain What is the major vascular territory covered by the ACA? MCA? PCA? (pp 465-466) __________ ______________________________________________________________________________ ______________________________________________________________________________ 19. What regulates cerebral perfusion?(p 465) ____________________________________________ Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 4 First Aid Express 2016 workbook: NEUROLOGY 20. Lesions in PICA or AICA may result in vomiting, vertigo, nystagmus and ipsilateral Horner syndrome. The two can be distinguished because _________ (AICA/PICA) lesions cause loss of pain and temperature in the face and limbs, whereas _________ (AICA/PICA) lesions cause paralysis of the face and pain/temperature loss in the face only. ________ (AICA/PICA) lesions cause decreased corneal reflex, whereas _______ (AICA/PICA) lesions cause loss of gag reflex, as well as dysphagia and hoarseness. (p 467) 21. On CT, an epidural hematoma _______________ (does/does not) cross suture lines, but a subdural hematoma _______________ (does/does not) cross suture lines. (p 469) 22. A 50-year-old woman presents to the emergency department with a headache and numbness on the right side. Her speech is difficult to understand, and her mouth droops when talking. Noncontrast CT of the head shows bright areas. Should tPA be administered? Why or why not? (pp 469-470) )______________________________________________________________________ 23. What are risk factors for development of idiopathic intracranial hypertension (pseudotumor cerebri)? (p 471) _______________________________________________________________ _____________________________________________________________________________ 24. What are the symptoms of normal pressure hydrocephalus? (p 471) _______________________ ______________________________________________________________________________ 25. Which spinal nerves exit the intervertebral foramina above the corresponding vertebra? Which spinal nerves exit the foramina below the corresponding vertebra? (p 472) ___________________ ______________________________________________________________________________ 26. At what spinal level is a lumbar puncture usually performed? (p 472) ________________________ 27. Using the following chart, compare and contrast the characteristics of upper and lower motor neuron lesions. (p 473) Characteristic UMN Lesion LMN Lesion Atrophy Babinski reflex Clasp knife spasticity Fasciculation Reflexes Spastic paralysis Tone Weakness Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 5 First Aid Express 2016 workbook: NEUROLOGY 28. For each lesion in the images below, identify the motor deficit and associated disease(s). (p 474) A. B. D. E. C. F. G. A. ____________________________________________________________________________ B. ____________________________________________________________________________ C. ____________________________________________________________________________ D. ____________________________________________________________________________ E. ____________________________________________________________________________ F. ____________________________________________________________________________ G. ____________________________________________________________________________ 29. Match these commonly tested reflexes to their nerve roots: (p 476) _____ A. Biceps 1. L4 _____ B. Patella 2. C5 _____ C. Triceps 3. S1 _____ D. Achilles 4. C7 Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 6 First Aid Express 2016 workbook: NEUROLOGY 30. What structures pass through the superior orbital fissure? (p 478) __________________________ 31. Fill in the following chart describing the cranial nerves. (p 479) CN I II Name Olfactory Optic III Oculomotor IV Trochlear V Trigeminal VI Abducens VII Facial VIII Vestibulocochlear IX Glossopharyngeal X Vagus XI Accessory XII Hypoglossal Function Copyright © 2016 by MedIQ Learning, LLC Type (Sensory/ Motor/Both) Location in Brainstem All rights reserved v1.0 page 7 First Aid Express 2016 workbook: NEUROLOGY 32. Which CNs mediate the pupillary response? (p 479) ____________________________________ 33. If there is a lesion in CNs V and VII, which reflex is impaired? (p 479) _______________________ 34. Which CNs mediate the gag reflex? (p 479) ___________________________________________ 35. With a lesion in CN X, the uvula deviates _______________ (toward/away from) the side of the lesion. (p 480) 36. With a lesion in CN XI, the head turns ________ (toward/away from) the side of the lesion. (p 480) 37. With a lesion in CN XII, the tongue deviates _______________ (toward/away from) the side of the lesion. (p 480) 38. What structures pass through the cavernous sinus? (p 481) ______________________________ ______________________________________________________________________________ OPHTHALMOLOGY 39. Where is the obstruction in open/wide angle glaucoma? Where is the obstruction in closed/narrow angle glaucoma? Which one is painful? (p 484)________________________________________ ______________________________________________________________________________ ______________________________________________________________________________ 40. Horner syndrome is associated with which three symptoms? (p 487) _______________________ ______________________________________________________________________________ 41. Referring to the image, which cranial nerve and muscle are tested with each movement? (p 488) Line A-B: _______________________________________________________________ Line C-D: _______________________________________________________________ Line E-F: _______________________________________________________________ Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 8 First Aid Express 2016 workbook: NEUROLOGY 42. Identify the type of visual field defects in the image. (p 489) 43. Horizontal diplopia develops in a 26-year-old woman with multiple sclerosis. Examination reveals she cannot adduct her left eye past midline and has a left-beating nystagmus in her right eye when looking to the right. However, her left eye can adduct during convergence. Where is the lesion most likely located? (p 490) ____________________________________________________________ ______________________________________________________________________________ PATHOLOGY 44. Match the type of dementia with its defining characteristic. (p 491) _____ A. Alzheimer disease _____ B. Creutzfeldt-Jakob disease _____ C. Frontotemporal dementia _____ D. Lewy body dementia 45. 1. 2. 3. 4. α-Synuclein defect Neurofibrillary tangles Prions Tau protein For each case, identify the type of seizure and its first-line treatment. (pp 494 and 500) A. A teenage boy suddenly stiffens, falls down, and experiences rhythmic jerking of his extremities lasting 1 minute. ___________________________________________________ B. A 7-year-old boy is referred to his primary physician for “behavioral problems” at school. He “spaces out” during class. EEG shows a 3-Hz spike-and-wave pattern. __________________ __________________________________________________________________________ C. A 1-year-old girl is brought to the emergency department because she had been shaking and unresponsive for 30 seconds. Her rectal temperature is 104ºF. ________________________ D. A 45-year-old man who suffered a concussion from a car accident has episodes of jerky movements of his left arm that he cannot control. He remembers the incident itself, but had blacked out afterward. ________________________________________________________ Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 9 First Aid Express 2016 workbook: NEUROLOGY 46. What are the main symptoms of a migraine? What mnemonic can you use to remember them? (p494) ________________________________________________________________________ ______________________________________________________________________________ ______________________________________________________________________________ 47. 48. For each case, identify the most likely neurocutaneous disorder. (p 495) A. A 6-month-old presents with her first seizure. Wood’s lamp examination shows several areas of hypopigmentation over her trunk and extremities. ________________________________ B. A 6-month-old has a port wine stain over his left eye and cheek, extending to the tip of his nose, with a sharp drop-off to normal-toned skin on the right side of his face. _____________ C. A 6-month-old has congestive heart failure. Imaging shows a cavernous hemangioma in the liver. ______________________________________________________________________ D. A 6-month-old has multiple hyperpigmented brown macules scattered over the trunk and upper extremities. _____________________________________________________________ For each case, identify the most likely brain tumor. (pp 496-497) A. A 49-year-old man presents with a 2-month history of morning headaches. CT of the head shows a heterogeneous-appearing mass with irregular borders crossing the corpus callosum. __________________________________________________________________________ B. A 40-year-old woman develops a small, well-circumscribed nodular-appearing lesion on her right frontal lobe. It appears to be attached to the skull. ______________________________ C. A 4-year-old boy presents with a 1-month history of morning headaches, abnormal gait, and dysmetria. Imaging shows an appearance in the posterior fossa. ______________________ D. A 7-year-old girl presents with bitemporal hemianopia. ______________________________ E. A 36-year-old woman presents with amenorrhea and “problems with peripheral vision” _____ ___________________________________________________________________________ PHARMACOLOGY 49. Match the drug with its indication for use. (pp 498-505) _____ A. Acute status epilepticus _____ B. Absence seizures _____ C. Chronic pain _____ D. Closed- / narrow-angle glaucoma _____ E. Induction of anesthesia _____ F. Insomnia _____ G. Opiate dependency _____ H. Seizure prophylaxis in pregnancy 50. 1. 2. 3. 4. 5. 6. 7. 8. Diazepam Ethosuximide Methadone Phenobarbital Pilocarpine Thiopental Tramadol Zolpidem Anesthetics with low blood and lipid solubility have __________ (fast/slow) induction, whereas anesthetics with high blood and lipid solubility have __________ (fast/slow) induction. (p 502) Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 10 51. First Aid Express 2016 workbook: NEUROLOGY What are the two clinical uses of dantrolene? (p 503) _______________________________ ______________________________________________________________________________ 52. Describe the mechanism of action for each drug commonly used to treat Parkinson disease. (pp 504-505) A. Benztropine ________________________________________________________________ B. Bromocriptine ______________________________________________________________ C. L-dopa D. Selegiline __________________________________________________________________ (levodopa)/carbidopa __________________________________________________ Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 11 First Aid Express 2016 workbook: NEUROLOGY Answers EMBRYOLOGY 1. Maternal diabetes (type 1). 2. “Cape-like” bilateral loss of pain and temperature sensation in upper extremities while fine touch is preserved. 3. The 3rd and 4th branchial arches. ANATOMY AND PHYSIOLOGY 4. A-4, B-3, C-1, D-2. 5. Schwann cells; oligodendroglia. 6. A-1, B-4, C-2 D-3. 7. Nonpolar/lipid-soluble substances cross rapidly (via diffusion); glucose and amino acids cross slowly (by carrier-mediated transport). 8. A-2, B-4, C-6, D-3, E-1, F-5, G-7. 9. Body-VPL, face-VPM. 10. Prolactin. 11. D1, facilitates, D2, inhibits. 12. Loss of dopamine inhibits the excitatory pathway and disinhibits (or excites) the inhibitory pathway. 13. Remember TRAPS: Tremor at rest, cogwheel Rigidity, Akinesia, Postural instability, Shuffling gait. 14. Substantia nigra; caudate nucleus; subthalamic nucleus. 15. A. B. C. D. 16. Wernicke; Broca. 17. A-7, B-12, C-6, D-9, E-5, F-3, G-1, H-13, I-11, J-2, K-4, L-8, M-10. 18. The ACA supplies the medial surface of the brain, which covers the leg area of the motor and sensory cortices. The MCA supplies the motor and sensory cortex of the face and arm. The PCA supplies the occipital cortex. 19. pCO2 Athetosis: Slow, writhing movements. Chorea: Sudden, jerky, purposeless movements. Dystonis: Sustained, involuntary muscle contractions. Myoclonus: Sudden, brief muscle contractions. Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 12 First Aid Express 2016 workbook: NEUROLOGY 20. PICA; AICA; AICA; PICA. 21. Does not; does. 22. This patient has had a stroke, and the bright areas on noncontrast CT indicate hemorrhage. Thus tPA should not be administered. 23. Risk factors for idiopathic intracranial hypertension (ICH) include being a woman of childbearing age, having excess Vitamin A, and the use of danazol. 24. Ataxia, dementia, and urinary incontinence. 25. Nerves C1-C7 exit above the corresponding vertebra; the others exit below the corresponding vertebrae. 26. In the L3-L4 or L4-L5 interspace. 27. Characteristic 28. UMN Lesion LMN Lesion Atrophy – + Babinski reflex + – Clasp knife spasticity + – Fasciculation – + Reflexes Spastic paralysis ↑ + ↓ – Tone ↑ ↓ Weakness + + A. Lower motor neuron lesions only, attributable to destruction of anterior horns; poliomyelitis and Werding-Hoffmann disease. B. Random and asymmetric lesions due to demyelination; multiple sclerosis. C. Combination of upper and lower motor neuron deficits with no sensory deficit; amyotrophic lateral sclerosis. D. Complete occlusion of anterior spinal artery with sparing of dorsal columns and tract of Lissauer. E. Degeneration of dorsal roots and dorsal columns; tabes dorsalis. F. Damage to the crossing fibers of the corticospinal tract; syringomyelia. G. Demyelination of dorsal columns, lateral corticospinal tracts, and spinocerebellar tracts; vitamin B12 neuropathy, vitamin E deficiency, and Friedrich ataxia. 29. A-2, B-1, C-4, D-3. 30. CN III, CN IV, CN V1, CN VI, ophthalmic vein, sympathetic fibers. Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 13 First Aid Express 2016 workbook: NEUROLOGY 31. CN I II Name Olfactory Optic Function Smell Sight III Oculomotor Extraocular eye movements Pupillary constriction Eyelid opening IV Trochlear V Trigeminal VI Abducens Extraocular eye movements Facial sensation Muscles of mastication Extraocular eye movements Type (Sensory/ Motor/Both) Sensory Sensory Location in Brainstem In cerebrum Midbrain Motor Midbrain Motor Midbrain Both Pons Motor Pons Both Pons VII Facial Muscles of facial expression Taste from anterior two thirds of tongue Lacrimation, salivation Eyelid closing Stapedius muscle in ear VIII Vestibulocochlear Hearing, balance Sensory Pons Glossopharyngeal Taste from posterior third of tongue Swallowing, palate elevation Salivation Chemo- and baroreceptors of the carotid body and sinus Both Medulla X Vagus Taste from epiglottic region Swallowing, palate elevation Midline uvula Muscles of pharynx and larynx Parasympathetics to visceral organs Aortic arch chemo- and baroreceptors Both Medulla XI Accessory Head turning Shoulder shrugging Motor Medulla XII Hypoglossal Tongue movement Motor Medulla IX Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 14 First Aid Express 2016 workbook: NEUROLOGY 32. CNs II and III mediate the papillary response. 33. The corneal reflex is impaired if a lesion occurs in CNs V and VII. 34. CNs IX and X mediate the gag reflex. 35. The uvula deviates away from the side of the CN X lesion. 36. There is weakness turning the head away from the CN XI lesion. 37. The tongue deviates toward the side of the CN XII lesion. 38. CN III, CN IV, CN V1, CN V2, CN VI, sympathetic fibers, internal carotid artery. OPHTHALMOLOGY 39. Open/wide angle glaucoma is due to obstructed outflow. Closed/narrow angle glaucoma is due to obstructed flow between iris and lens. Closed/narrow angle glaucoma is painful. 40. Ptosis, anhidrosis, miosis. 41. Line A-B (SR-SO): assesses CN III—all but the superior oblique and the lateral rectus. Line C-D (IO-IR): assesses CN IV—the superior oblique. Line E-F (LR-MR): assesses CN VI—the lateral rectus. 42. 43. Her left eye can adduct during convergence but not during right lateral gaze because the oculomotor nerve itself works perfectly, but the connection between the abducens nuclei and the oculomotor nuclei is impeded. The “message” to look right does not reach the left medial rectus, causing the right eye to beat leftward because of the dysconjugate image. (If you are still confused, now would be an excellent time to review INO.) Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0 page 15 First Aid Express 2016 workbook: NEUROLOGY PATHOLOGY 44. A-2, B-3, C-4, D-1. 45. A. B. Generalized tonic-clonic (grand mal) seizures. Treat with phenytoin, carbamazepine, or valproic acid. Absence seizures. Treat with ethosuximide. C. Febrile seizures. Do not use antiepileptics. Give acetaminophen for the fever. If it appears to be a simple febrile seizure (1 seizure/illness; no previous febrile seizures), recurrence of seizure is unlikely. D. Simple partial seizures with secondary generalization. Virtually any antiepileptic drug can be used; the most common are phenytoin, carbamazepine, levetiracetam, and valproic acid. 46. The main symptoms of migraines are unilateral, pulsating pain with nausea, photophobia, or phonophobia. This pain is usually disruptive to everyday activity. The mnemonic that one can use to remember these symptoms are POUND (Pulsatile, One-day duration, Unilateral, Nausea, Disabling). 47. A. B. C. 48. D. Tuberous sclerosis. Sturge-Weber syndrome. Cavernous hemangiomas can occur in isolation, but are associated with von Hippel-Lindau disease. Neurofibromatosis type 1. A. B. C. D. E. Glioblastoma multiforme. Meningioma. Medulloblastoma. Craniopharyngioma. Prolactinoma (pituitary adenoma) PHARMACOLOGY 49. A-1, B-2, C-7, D-5, E-6, F-8, G-3, H-4. 50. Fast; slow. 51. Malignant hyperthermia and neuroleptic malignant syndrome. 52. A. Inhibits acetylcholine, which preferentially excites the inhibitory pathway over the excitatory pathway. The net effect of acetylcholine is inhibition of the thalamus. B. Dopamine receptor agonist. C. Inhibits peripheral breakdown of L-dopa, which crosses the blood-brain barrier and is converted to dopamine in the brain. The net effect is an increase of dopamine in the brain milieu. D. Inhibits MAO-B enzyme, resulting in less breakdown of dopamine in the CNS. Copyright © 2016 by MedIQ Learning, LLC All rights reserved v1.0